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Message to the STCA Membership:

Updated 3-February-2008

Update on Research into the Genetic Cause of Hereditary Ataxia in American Staffordshire Terriers - January 2008

This is a brief update to let you know that we are continuing to make good progress with our research into Hereditary Ataxia in American Staffordshire Terriers. The last update I gave reported that we had established linkage between the disease and a limited region of a chromosome. This region was large, too large to identify one particular gene that could be causing the disease. In order to address this problem, we submitted a grant proposal to the AKC Canine Health Foundation requesting funding for the next step in our research - fine mapping of the linked region and sequencing of candidate genes. The grant has been approved and we expect to have the funding available to us shortly. In the interim, with the support of the STCA, we have genotyped a much larger number of dogs (nearly 100) and added in closely spaced markers over the linked region. As a result, we have been able to narrow down the area of linkage and we are getting ready to sequence one particular gene that could be linked to the disease. Over the next year we will work on the possible candidate genes in the linked region with the hope that they will lead us to the causative mutation and hence to a genetic test. If you know anyone with an affected dog, we would love to hear from them, and if you would like to make a donation to support the research, please contact Faith Beiser. Thank you all for your continued support and we hope that 2008 will be a productive year.

Natasha Olby (Natasha_olby@ncsu.edu)

Dear members of the STCA,

It was a great pleasure for me to meet many of you and to see your beautiful dogs at the national show in Texas. Thank you so much for inviting me and listening to my presentation. Thank you in particular to Faith Beiser; she is a wonderful advocate for the STCA. The most recent set of sampling protocol sheets and consent forms can be downloaded from the health page. Please note that Jeanne Burr no longer works at NCSU and Pragna Mehta has replaced her as a contact person and technician on the project. Pragna's e mail address is pragna_mehta@ncsu.edu and telephone number is 919 513 7235.

Please do not hesitate to contact me Natasha_olby@ncsu.edu) or Pragna if you have any questions.

Sincerely,

Natasha Olby

And, our many thanks to Dr, Olby.
Now Available!
Presentation overview by Guest Speaker Dr. Natasha Olby
on Cerebella Ataxia in AmStaffs from the STCA National Specialty
on 10-19-2006 at Triple Crown Dog Academy
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Important!!!!

STCA 2006 Follow Up Health Survey
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Information for submitting blood samples:

Health Committee AmStaff Print Fundraiser! Limited Edition collectors item, numbered, only 100 available. Cost of print is $55, plus $6 shipping. For more information and to Order, click here!

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STCA Health Committee Updates

Last updated June 14, 2006.

Note from Dr. Nastasha Olby

I have nearly completed the full genome screen in 48 dogs: all closely related including many affected dogs. We will be finishing off the screen in these dogs and doing the linkage analysis this summer and we hope to have some news by the national, one way or another. Of course, this news could be that we were unable to establish linkage, but that is still useful information for deciding the best way forwards.

I would also like everyone to know that I feel that the success of this project and my professional integrity require that I continue to maintain the confidentiality that I have promised all the people who have taken part. I cannot tell people which pedigrees are likely to carry the problem at this time. I hope that the development of a genetic test will solve this serious problem for the breed.

--
Natasha Olby Vet MB PhD
Diplomate ACVIM (Neurology)
Associate Professor of Neurology
College of Veterinary Medicine
North Carolina State University
4700 Hillsborough St
Raleigh
NC27606

Wobbly dogs: an explanation of the significance of different signs
By Dr. Nastasha Olby

As nearly everyone involved with American Staffordshire terriers knows, the breed has been afflicted with an inherited disease that causes progressive incoordination (ataxia). Different areas of the nervous system control coordination and it is important to understand that disease of any type (e.g. cancer, infection or degeneration) affecting these areas of the nervous system can cause incoordination. The important areas of the nervous system for control of coordination are the cerebellum, the vestibular system and the spinal cord, and all three areas interconnect. This article attempts to briefly describe and explain the different signs that can occur when disease affects these areas. The typical diagnostic approach to a dog presenting with ataxia is also explained.

a) The cerebellum is responsible for controlling the rate, range and force of movements and when it fails, animals become incoordinated (ataxic) and tend to stand with their feet far apart to stabilize themselves. They may develop a high stepping gait (also called dysmetria or goose stepping because of its appearance), and they find it difficult to judge a movement and so develop a sign called intention tremor. This is most obvious when an animal is really intent on a task such as eating and their head will literally tremor from side to side making it difficult for them to accomplish the task. The classic signs of cerebellar disease can therefore be summarized as wide based stance, ataxia characterized by dysmetria, and intention tremor, and the signs are readily recognized by veterinarians. As the cerebellum does not control strength animals with cerebellar disease are not weak. However, the cerebellum does connect closely with the vestibular system and cerebellar disease can cause signs of vestibular disease (described below) in addition to the more typical cerebellar signs. This can produce some confusion when evaluating animals.
b) The vestibular system involves both a sensory system in the inner ear and its projections to the brainstem and cerebellum. The vestibular system compares information generated as a result of head movements and the force of gravity from both inner ears and aligns the head and body in a normal posture against gravity. It also ensures that your eye movements respond to head movements in such a fashion that you can fix on an image when your head is moving. Signs of vestibular disease are typically worse on one side than the other (except in the case of degenerative diseases), for example, a middle ear infection can spread to the inner ear and affect the information coming in from that side. Typical signs include a head tilt (that can be dramatic); flicking of the eyes from side to side or up and down (nystagmus); abnormal position of the eyes (one eye looks down while the other looks straight ahead: strabismus); vestibular ataxia (incoordination) characterized by leaning, falling and circling to the side of the head tilt (the body is trying to align itself with respect to gravity based on wrong information); and nausea. If the part of the vestibular system in the brainstem is involved, you can also see weakness, and changes in consciousness, sometimes leading to coma.
c) The spinal cord conveys sensory information on the position of the limbs to the brain. Damage to the spinal cord interrupts this information causing the final form of ataxia: sensory ataxia. Affected animals will cross their legs when walking, giving them a drunken appearance, and may walk on the back of their paws because they fail to correct paw position properly. Typically the animals will also be weak.

My usual approach as a neurologist to a dog presenting with ataxia (incoordination) is to complete a careful neurological examination and from that distinguish between cerebellar, vestibular and sensory ataxia. This allows me to localize the problem to one area of the nervous system and I then use other information (e.g. how quickly did the signs come on, are they getting better or worse, how old is the animal, what other medical history does it have), to generate a list of possible causes. This in turn allows me to recommend further diagnostic tests (which in neurology often means imaging the brain using a CT scanner or MRI, and taking a sample of spinal fluid). Once these tests have been run we arrive at a diagnosis and determine the most appropriate treatment.

The inherited neurodegenerative disease that has emerged as a problem in Amstaffs affects the cerebellum, but involves in particular the portion of the cerebellum that connects to the vestibular system. We therefore see a mix of vestibular signs (e.g. nystagmus) and cerebellar signs. This can lead to a failure to consider this as a possible diagnosis (it does not appear like the most classic cerebellar disorder) and to the possibility of pure vestibular diseases representing the more sinister diagnosis of hereditary ataxia.

We do commonly see vestibular syndromes that are much less concerning in Amstaffs. One is 'idiopathic vestibular syndrome.' This is a disease of the inner ear for which the underlying cause is unknown but the brain itself is not involved. This disease causes very sudden onset of a head tilt, falling, nausea and nystagmus: the signs can be very dramatic and it is typical for an owner to think their dog has had a stroke. There is no curative treatment and in general the signs resolve or greatly improve on their own within 2 - 4 weeks. We will usually check a thyroid panel and blood pressure in affected dogs, but they are often normal and imaging of the brain and CSF analysis are normal. We also see infections of the middle ear (otitis media/interna) cause very similar signs of head tilt, falling and nystagmus. Often the animal's ear is painful. This can sometimes be diagnosed by a very good examination of the ear, but it can be difficult to see as far as the middle ear in dogs and a CT scan of the head may be necessary. This disease must be treated with antibiotics and sometimes surgery is indicated to remove all the infection and inflammation from the middle ear.

Both of these vestibular diseases are sometimes associated with paralysis of muscles of the face on the same side as the head tilt: called facial paralysis. This results in drooping of the face, inability to close the eye, accumulation of food in the cheek and drooling, all on the same side as the head tilt. In the case of otitis media, the nerve running to the facial muscles also runs through the middle ear and can be coincidentally affected by the infection. In the case of idiopathic vestibular syndrome, sometimes we see idiopathic facial paralysis at the same time. In this latter instance we suspect that the unidentified disease process affecting the vestibular system is also affecting the facial nerve, although more usually these idiopathic disease occur separately. Importantly, facial paralysis is not a component of hereditary ataxia.
If you notice your dog is incoordinated, the first step is to take them to your veterinarian so that they can do a neurological examination. If they suspect a spinal cord or brain problem, they may then decide to refer you to a neurologist to complete the more specialized diagnostic tests and get a definitive diagnosis from which the best treatment can be planned. A full list of neurologists is available on the following website: www.acvim.org. If you are not interested in a full neurological work up, they may be able to discuss the possible causes and the treatment and prognosis (outcome) for each of these diseases to help you to make a decision about the next step.

Emerging Ataxia in the American Staffordshire Terrier
Natasha Olby
Updated January 11, 2004

Amstaff Ataxia FAQs

There has recently been some healthy debate on the topic of Amstaff ataxia and possible causes for this devastating problem. I thought it would be useful to address some of the frequently asked questions on the web site.

1. Is Amstaff ataxia a canine form of Mad Cow Disease?

The answer to this question is NO. Here are the reasons why:

1. Mad Cow disease, also called bovine spongieform encephalopathy, produces classic changes in the brain that can be seen when sections of the brain are examined with a microscope. The most obvious change is an appearance of holes within neurons, and indeed this is what gives the disease its scientific name of spongieform (sponge like) encephalopthy (brain disease). This appearance is the hall mark of the disease and is present in all forms of the disease (e.g. the human, feline and ovine). All of the diseases can be lumped together and called the "transmissable spongieform encephalopathies". If there are no "holes" in the neurons it is not a spongieform encephalopathy. In Amstaff ataxia there are no holes in the neurons.
2. In Mad Cow disease the changes in the brain are found in several regions, but in particular in the gray matter of the cerebrum and cerebellum. In Amstaff ataxia, the changes are only found in the cerebellum, and even more specifically in the 3 outer layers of the cerebellum.
3. We have shown statistically that this disease is inherited.

1. If Amstaff ataxia is inherited, why do we only see occasional affected dogs and why are several generations skipped sometimes?

1. Based on our statistical analysis of pedigrees, we believe that this disease is a recessive disease. By this we mean that an animal must have 2 copies (one from each parent) of the abnormal (mutated) gene to cause signs of the disease. If they only have one copy of the gene they appear to be normal. As a result, apparently normal parents, each of which have one gene for the disease (making them hidden carriers of the disease) can produce a mix of affected and normal pups if bred with other carriers. This means that they can be bred over and over again without problem, it is only when they are crossed with another carrier that you can get affected offspring. Even then there is only a 25% chance that a dog will inherit copies of the abnormal gene from both parents.
2. To add to this already confusing picture, affected dogs may not develop signs until they are over 8 years of age (most start to show signs between 4 and 6 years of age) and so the breeder may well not know that they have produced affected dogs as they can be so widely scattered by the time they show signs.
3. Finally, the signs shown by dogs can vary in severity and because this is a new and relatively rare disease, it may not be recognized by the veterinarian. As there is no simple diagnostic test that confirms the disease at present, this makes diagnosis even more difficult.
4. All of this factors conspire to make it difficult to know precisely which dogs in a litter are affected and make it difficult to be certain of the mode of inheritance. The only way to be 100% sure is to do test breedings between known affected dogs and keep all the offspring and watch them over at least 8 years: truly a slow and expensive process.

1. Does diet play a role in Amstaff ataxia?

There has been a lot of discussion on the role of diet, and in particular on the role of raw diets in the pathogenesis of this disease. We did look at this quite carefully and even measured levels of vitamin E in the blood of affected dogs to see if there was a deficiency (a similar disease in people can be caused by vit E deficiency as a result of a mutation that causes abnormal processing of vit E). We found no evidence of diet playing role: the dogs we have seen are on a wide variety of different diets and had excellent blood levels of vitamin E.

2. Could the disease be caused by a toxin such as organophosphate toxicity?

This is another possible cause that we looked at carefully. The pathology (changes) that we see in the brain of affected dogs are not consistent with a toxicity. Moreover, affected dogs come from all over the world so it is difficult to imagine that they have all been exposed to the same toxins, and in fact the only thing that they have in common is their lineage. There is always the possibility that the affected dogs have a genetic mutation that makes them more susceptible to a toxin that we have not identified as yet, but it seems unlikely.

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The Problem
As with all pure bred dogs, American Staffordshire terriers exhibit some health problems that are most likely the result of genetic defects. Recently, the Staffordshire Terrier Club of America's health committee has noticed that there are increasing numbers of dogs developing a drunken, staggering gait and balance problems as they get older. This is a particularly serious disease, as dogs do not exhibit signs until well into adulthood, after they have been bred. Current research indicates that this is most likely a genetic disorder.

The Symptoms
This disease affects a particular part of the brain called the cerebellum. The cerebellum is closely associated with the vestibular system and together these parts of the brain control coordination of movements. There are many different diseases that can affect these areas of the brain and therefore produce similar signs. Infection of the middle ear, trauma, viral infections, tumors, autoimmune disorders and bacterial disease can also cause vestibular and cerebellar signs. However, the order and rate in which signs develop in dogs with this particular syndrome are characteristic of the disease. Both male and female dogs are affected and signs may start from 2 - 5 years of age.

Initially, the signs are very subtle and include clumsiness and an occasional sway of the body when making sudden movements. Agility dogs, for example, may develop problems with a particular obstacle that they could previously negotiate without difficulty. Dogs may trip and fall when negotiating steps or moving quickly. As the disease progresses, the clumsiness becomes more pronounced: dogs may fall over when they shake their head and often have an obvious body sway when they move their head quickly. Male dogs may lose their balance while lifting their legs to urinate. When moving in a controlled fashion in a straight line they may still look normal, but when turning corners, chasing balls, and rushing for their food, the mistakes become obvious.

If the dog's eyes are watched carefully, it is noticed that sudden movements of the head cause rapid flicking of the eyes (nystagmus), either from side to side, round in circles or vertically. Rolling the dog onto its back is the most reliable way of eliciting these eye movements and they usually last from a few seconds to about one minute.

Other early signs of the disease may include sudden episodes of collapse, with stiffening of all 4 legs, the neck and the body that last for a few seconds. With time, the dogs have more and more difficulty walking because of lack of coordination of their legs rather than weakness. The AmStaf may also lose weight as the dog has balance difficulties reaching his food bowl.

Please remember that we are very early in our data collection process. The age of onset, symptoms, and progression varies from dog to dog. The above descriptions are general signs of hereditary ataxia. Your dog does not have to have every characteristic listed in order to be considered affected. The one similarity that all the dogs have shared thus far has been balance problems.

The Prognosis
The progression of the disease varies from dog to dog. For many AmStafs, the signs develop slowly. The STCA health committee considers hereditary ataxia a "deadly" disease since most AmStafs cannot live out a normal lifespan and must be humanely euthanized. Most are not able to function past the age of 7 or 8. One note: if anesthesia is performed for some reason (e.g. a routine dental), it may accelerate deterioration. We have witnessed dogs with minimal signs waking up from anesthesia extremely unbalanced with obvious nystagmus. These signs improve somewhat in the days after anesthesia, but the dog does not recover to the level it was at prior to anesthesia.

The Diagnosis
If you have an AmStaf exhibiting any neurologic signs, (even those considered to be the result of an ear infection or vascular event like a "stroke") it is HIGHLY recommended that you seek the advice of a veterinary neurologist. If you need assistance or advice, please contact Dr. Natasha Olby and her research team at the below numbers. A complete neurologic workup is most helpful in ruling out the many other types of disease processes that can produce the same symptoms. Tests that may include a complete blood cell count, a biochemistry panel, a computed tomography (CT) scan or magnetic resonance imaging (MRI) to evaluate the brain and middle ears for evidence of a mass (such as a brain tumor) or infection in the middle ears, and cerebrospinal fluid analysis (spinal tap) to rule out encephalitis. In dogs with this syndrome, all test results are normal. Post mortem examination by a pathologist is one of the most helpful diagnostic tools we have. Data gathering is critical to halting the spread of this terrible disease.

The Research
Dr. Natasha Olby has evaluated several of these dogs at North Carolina State University veterinary teaching hospital and she is working to identify the genetic defect causing this problem. Our aim is to develop a test for the disease to identify affected dogs prior to breeding them. Similar syndromes occur in people and the genetic defects that cause many of them have been identified. We are therefore starting our research by looking for the same genetic defects described in humans.
We are interested in information from dogs who are currently exhibiting the problem as well as dogs thought to have died from this disorder (or a similarly progressive neurologic disease). For those of you who have had dogs die some time ago, your information is still very valuable to us. If you suspect that your dog has this problem, please contact Dr. Olby's research team by telephone: 919 513 7235 (ask to speak to Pragna Mehta) or by e mail (pragna_mehta@ncsu.edu). They will need a description of your dog's signs, with a videotape if at all possible. They will be able to tell you where the nearest veterinary neurologist is located if they feel that your dog would benefit from a full diagnostic work-up. If Dr. Olby suspects that your dog has this disease, we would appreciate your help in solving the problem. All information is strictly confidential and will be maintained by Dr. Olby and her team. No one else (including the health committee) will have access to raw data. We are trying to determine how this disease is inherited and the researchers may ask you for a copy of your dog's pedigree: this information will be kept completely confidential at the vet school. Blood samples may also be requested from your vet and would enable Dr. Olby to perform genetic analysis. These will be kept solely for the purposes of this research project.

Investigator: Natasha Olby Vet. M.B., Ph.D., Diplomate ACVIM (Neurology)

If you would like to help but you do not have an affected dog, you can contribute directly to the cause via donations. Checks can be sent directly to the AKC Canine Foundation, 251 W. Garfield Rd. Suite 160, Aurora, Ohio 44202-8856. Please include on the memo portion of the check "American Staffordshire Terrier Donor Advised Fund". Donations are tax deductible and can only be used in health research for the AmStaf. Please do not contribute directly to the health committee.

The Recommendations
Evidence indicates that this disease is on the rise, and that it may be present in Europe as well. We are working with our counterparts there to gather more information. Until genetic testing is available, we can only make recommendations for breeders to avoid spreading this disease. Because ataxia is a late onset disease, do not breed young stock. Do not breed ANY dog with neurologic symptoms (this includes those thought to be caused by ear infections and strokes) unless it has been definitively diagnosed to have a non-hereditary disease process by a neurologist. If you suspect a problem with your dog, please participate in our research project. It is completely confidential, and it is the only way we can battle this terrible problem.

Update on Cerebellar Cortical Degeneration (Hereditary Ataxia) in American Staffordshire Terriers
January, 2003

Since we started investigating ataxia in American Staffordshire terriers, we have recruited 44 affected American dogs to the study and are working with researchers in Europe where the problem is unfortunately also well-recognized. Our initial aims are to establish how (and whether) the disease is inherited, to describe the full spectrum of signs that it causes, to characterize the pathological changes in the brain and to collect DNA (from blood samples) from affected and unaffected dogs. Our secondary aims are to use this information to hunt for the genetic defect that is causing the disease, and if we achieve this, to develop a test for the disease that could be used prior to breeding.

Progress
Owners and breeders of affected dogs have been extremely supportive of this work. This is a terrible disease and those that have watched a dog progress from the first signs of the disease (occasional staggering) to the more severe stages (dramatic swaying and staggering, with frequent hard falls and difficulty taking even a single step) are committed to trying to wipe it out. Unfortunately, we have confirmed that the disease is inherited, most likely by an autosomal recessive trait (in other words, dogs can carry one copy of the gene and they do not show any signs of the disease, it is only when a dog has two copies of the gene that the disease manifests itself), and the evidence so far suggests that the gene is widely distributed in the breeding population of American Staffordshire terriers. As the signs of this disease come on anywhere from 2 to 8 years of age, it has been extremely difficult tracing littermates to affected dogs in order to determine how many dogs in each litter are affected, information that is vital for confirmation of the mode of inheritance. We really need to hear from anyone who has a dog that is showing signs of the disease (staggering, wobbly), and anyone who has a normal dog, but knows that a littermate is affected…we need to know about the normal littermates as well.

The signs of the disease were described in our initial notice posted by the health committee. We are now suspicious that the signs are coming on earlier (around 2 years of age) and with greater severity (progressing over 6 - 24 months instead of 4 - 6 years) in the offspring of affected dogs. Although there is no diagnostic test that can truly confirm the presence of this disease other than an autopsy, we have found that the cerebellum, (the part of the brain that controls coordination) is small on magnetic resonance (MR) images in affected dogs. Unfortunately, MR images are not useful for detecting the disease prior to onset of signs so cannot be used to detect the disease in apparently normal dogs prior to breeding from them.

With the help of owners, breeders and their veterinarians, we have collected DNA from a lot of affected and unaffected dogs and we are getting to the point where we can start to do the genetic analysis. Unfortunately, we are now limited by a lack of funds: this type of work costs thousands of dollars, and in the current financial climate, that money is just not available. We are working hard to try to find the kind of funding that we need, and in the meantime we are continuing to collect DNA, pedigree information and to confirm the presence of the disease when dogs die by performing an autopsy (this last stage is critical to the research as it is currently the only way to definitively confirm the presence of the disease). We would like to thank all the dedicated owners and breeders of AmStaffs for the help that they have given us so far, and to urge those who suspect that they have an affected dog to contact us. We will maintain the anonymity of affected dogs and their owners. We can be contacted at: technician Pragna Mehta: e mail address is: Pragna_mehta@ncsu.edu, or Natasha Olby Vet.M.B., Ph.D., e-mail addres is Natasha_Olby@ncsu.edu, telephone: 919-513-8286, fax: 919 513 6714.

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Orthopedic Foundation for Animals

The Orthopedic Foundation for Animals (OFA) web site offers a database of information concerning orthopedic and genetic diseases of animals. The new search features provide easy access to find information on dogs in the database and their tested relatives. To start your search, use the QUICK Search box at the top of the page. OFA results are also published in the STCA Quarterly Magazine.

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